Myositis-associated Interstitial Lung Disease: Predictors of Failure of Conventional Treatment and Response to Tacrolimus in a US Cohort

2017 ◽  
Vol 44 (11) ◽  
pp. 1612-1618 ◽  
Author(s):  
Niharika Sharma ◽  
Michael S. Putman ◽  
Rekha Vij ◽  
Mary E. Strek ◽  
Anisha Dua
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Clinical Characteristics ◽  
Conventional Treatment ◽  
Case Reports ◽  
Response To Therapy ◽  
Disease Modifying Antirheumatic Drugs ◽  
Antirheumatic Drugs ◽  
Small Series ◽  
The Mean

Objective.Patients with myositis-associated interstitial lung disease (MA-ILD) are often refractory to conventional treatment, and predicting their response to therapy is challenging. Recent case reports and small series suggest that tacrolimus may be useful in refractory cases.Methods.A retrospective cohort study of patients with MA-ILD comparing clinical characteristics between those who responded to or failed conventional treatment. In those who failed conventional treatment and received adjunctive tacrolimus, response to tacrolimus was measured by the improvement in myositis, ILD, and change in the dose of glucocorticoids.Results.Thirty-one of 54 patients (57%) responded to conventional treatment based on the predefined variables of improvement in myositis and/or ILD. Patients with polymyositis (PM)-ILD were more likely to respond to conventional treatment than those with dermatomyositis (DM)-ILD (67% vs 35%, p = 0.013). Twenty-three patients failed conventional treatment, 18 of whom subsequently received adjunctive tacrolimus. Ninety-four percent had improvements in ILD and 72% showed improvement in both myositis and ILD. The mean doses of prednisone decreased from baseline by 65% at 3–6 months (p = 0.002) and 81% at 1 year (p < 0.001).Conclusion.Patients with PM-ILD were more likely to respond to conventional treatment than patients with DM-ILD, but clinical characteristics and serology did not otherwise predict response to therapy. A majority of patients with MA-ILD refractory to conventional therapy improved while receiving tacrolimus and were able to decrease their dose of both glucocorticoids and other disease-modifying antirheumatic drugs.

scholarly journals AB0277 PREVALENCE OF HEPATITIS MARKERS IN PATIENTS TREATED WITH BIOLOGICAL DISEASE-MODIFYING ANTIRHEUMATIC DRUGS: RESULTS OF THE TUNISIAN REGISTRY BINAR

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1437.1-1438
Author(s):  
A. Fazaa ◽  
H. Boussaa ◽  
S. Miladi ◽  
K. Ouenniche ◽  
L. Souabni ◽  
...  
Keyword(s):  
Hepatitis B ◽  
Liver Function ◽  
Disease Activity ◽  
Liver Function Tests ◽  
Hbv Dna ◽  
Disease Modifying Antirheumatic Drugs ◽  
Antirheumatic Drugs ◽  
The Mean ◽  
Disease Modifying ◽  
Hbs Ag

Background:In the recent decades, biological disease-modifying antirheumatic drugs (bDMARDs) have significantly improved management and quality of life in patients with rheumatoid arthritis (RA) and spondyloarthritis (SpA).However, bDMARDs have also a strong influence on the immune system, leading to a risk of serious infection. Reactivation of hepatitis B (HBV) and C (HCV) virus is one of the most redoubtable complications of these immunosuppressive agents.Objectives:The aims of this study were to determine the screening rate for hepatitis B and C before starting a biological treatment and to examine the prevalence of their markers in patients with RA or SpA.Methods:Our study evaluated all patients included in the Tunisian registry BINAR (Biologic National Registry) since 2018 who had RA (ACR/EULAR 2010) or SpA (ASAS criteria) aged with more than eighteen years old and receiving their first bDMARDs during the two past years.The following information were retrieved from the registry: demographic data on the patients, disease parameters, medication, HBV surface antigen (HBs Ag), antibody to HBs Ag (Anti HBs), antibody to HBV core antigen (Anti HBc), HBV-DNA, antibody to HCV (anti HCV) status and liver function tests (AST: aspartate aminotransferase; ALT:alanine aminotransferase).Results:A total of 298 patients was included, 111 men and 178 women, with a mean age of 49.2 ± 14.1 years old [18-79]. Among them, 58.7% were diagnosed with RA and 41.3% were diagnosed with SpA. The mean disease duration was 6.7±3.5 years [1-12] in patients with RA and 6.5±3 [1-12] in patients with SpA. The mean Disease Activity Score (DAS28) and Bath Ankylosing Spondylitis Disease Activity Index (BASDAI) were respectively of 4.9±1.5 [1-8] and 4.1±1.8 [0-9].Therapeutically, 167 patients (56%) were on Prednisone at a mean daily posology of 8.2±5.4 mg [4-60] and 70.3% on conventional synthetic disease modifying antirheumatic drug (csDMARD) in association with bDMARDs. It was about Tumor Necrosis Factor alpha antibodies (anti TNF a) in 87.9% of cases, Tocilizumab in 10.4% of cases and Rituximab in 5% of cases.A screening of HBV was performed in 286 patients (96%). Ag HBs was positive in two cases (0.7%), and anti-HBc was positive in 16 cases (6.4%) which indicate a prior HBV infection. Fifteen patients (6%) were immunized with positive anti HBs. HBV-DNA was measured in 177 cases (66.8%) and was positive in 15 patients (6%).HCV infection was searched in 282 patients (94.6%) and anti-HCV was negative in all cases.AST and ALT mean rates were respectively of 18.3 [2-108] and 17.9 UI/l [2-74]. A perturbation of these liver function tests was observed in 13 patients (4.4%).Conclusion:Screening for hepatitis B and C were performed respectively in 96% and 94% of our Tunisian patients before receiving any bDMARDs. This should be systematic to avoid HBV reactivation which can lead to fulminant hepatic failure with a severe prognosis.Disclosure of Interests:None declared

Clinical Characteristics and Genetic Analysis of Interstitial Lung Disease in Chinese Children (Genes and Interstitial Lung Disease)

2021 ◽  
Author(s):  
Mei-Xia Huang ◽  
Lu Qin ◽  
Fei-Zhou Zhang ◽  
Lei Wu ◽  
Jia-Hui Yu ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Clinical Characteristics ◽  
Clinical Manifestations ◽  
Failure To Thrive ◽  
Surfactant Protein ◽  
Chinese Children ◽  
Common Mutation ◽  
Onset Age ◽  
Surfactant Dysfunction

Abstract BackgroundMutation in the surfactant protein C gene (SFTPC) is a cause of interstitial lung disease (ILD). Our objective was to investigate the clinical characteristics, outcome and influencing factors of ILD in Chinese children with SFTPC mutations.MethodA total of 8 Chinese children with ILD heterozygous for SFTPC mutations that were treated in our hospital from January 2014 to December 2020 were included in our study. Candidate genes responsible for surfactant dysfunction were sequenced by next-generation sequencing. The clinical and genetic data were reviewed retrospectively.ResultsThe children’s onset age was before the age of 2 years, and one case was just after birth. The most significant clinical manifestations were cough, tachypnea, hypoxemia and failure to thrive. The most common mutation was p. lle73Thr, which accounted for 87.5% (7/8) of our patients. Four patients whose onset was within 3 months, including 3 children with CMV infection, died. Conclusionp. lle73Thr mutation of SFTPC was an important and common cause of ILD in the Chinese children. The clinical manifestations of ILD associated with this mutation are not specific. The severity and outcome of the disease may be affected by factors such as onset age and viral infection.

scholarly journals The King’s Brief Interstitial Lung Disease (KBILD) questionnaire: an updated minimal clinically important difference

2019 ◽  
Vol 6 (1) ◽  
pp. e000363 ◽  
Author(s):  
Aish Sinha ◽  
Amit Suresh Patel ◽  
Richard J Siegert ◽  
Sabrina Bajwah ◽  
Toby M Maher ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Minimal Clinically Important Difference ◽  
Scale Transformation ◽  
Global Rating ◽  
Longitudinal Assessment ◽  
Hrqol Questionnaire ◽  
Clinically Important Difference ◽  
The Mean ◽  
Small Change

IntroductionThe King’s Brief Interstitial Lung Disease (KBILD) is a 15-item validated health-related quality of life (HRQOL) questionnaire. The method of scoring the KBILD has recently changed to incorporate a logit-scale transformation from one that used raw item responses, as this is potentially a more linear scale. The aim of this study was to re-evaluate the KBILD minimal clinically important difference (MCID) using the new logit -transformed scoring.Methods57 patients with interstitial lung disease (17 idiopathic pulmonary fibrosis, IPF) were asked to complete the KBILD questionnaire on two occasions in outpatient clinics. At the second visit, patients also completed a 15-item global rating of change of health status questionnaire (GRCQ). The MCID was calculated as the mean of four different methods: the change in KBILD for patients indicating a small change in GRCQ, patients with a 7%–12% change in FVC, 1 SE of measurement of baseline KBILD and effect size (ES) of 0.3.ResultsThe mean (SD) KBILD total score for all patients was 55.3 (15.6). 16 patients underwent a therapeutic intervention. 36 patients reported a change in their condition on the GRCQ; 22 deteriorated, 14 improved and 21 were unchanged. There was a significant change in KBILD total score in patients reporting a change in GRCQ; mean (SD) 57.0 (13.6) versus 50.0 (9.7); mean difference 7.0; 95% CI of difference 3.0 to 11.0; p<0.01. The change in KBILD total score correlated with the GRCQ scale; r=−0.49, p<0.01. The mean KBILD total score MCID was 5. The MCID of KBILD domains were 6 for Psychological, 7 for Breathlessness and Activities, and 11 for Chest Symptoms.ConclusionThe KBILD is a responsive tool for longitudinal assessment of HRQOL in patients with ILD. The MCID of the KBILD total score is a 5-unit change.

Rheumatoid Arthritis-Associated Interstitial Lung Disease: Clinical Characteristics and Predictors of Mortality

Respiration ◽  
2019 ◽  
Vol 98 (5) ◽  
pp. 455-460 ◽  
Author(s):  
Charlotte Hyldgaard ◽  
Torkell Ellingsen ◽  
Ole Hilberg ◽  
Elisabeth Bendstrup
Keyword(s):  
Rheumatoid Arthritis ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Clinical Characteristics ◽  
Predictors Of Mortality
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